Background: Pulmonary sequestration (PS) is defined as nonfunctional tissue within lung, has separate arterial supply from lung itself and does not have any communication with the tracheobronchial tree. PS is divided into two types which are intralobar pulmonary sequestration (ILS) and extralobar pulmonary sequestration (ELS). PS is a rare congenital malformation of the lungs which is composed of 0.15% to 6.4% of all congenital lung malformation. PS is commonly located in the left lobes and its incidence, especially the incidence of ILS, in the pediatric population is higher than in the adult population. Diagnosis of intralobar pulmonary sequestration which is in the right lower lobe in the third decade of life is a rare situation. We present a case of 37-year-old man diagnosed with ILS located in the right lower lobe which has its own arterial supply directly from descending aorta. This rare case was treated with right lower lobectomy just after the coil embolization of its arterial supply with the help of interventional radiology.
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